TY  - CHAP
M1  - Book, Section
TI  - Pulmonary Alveolar Proteinosis Syndrome
A1  - Trapnell, Bruce C.
A1  - McCarthy, Cormac
A1  - Carey, Brenna
A2  - Grippi, Michael A.
A2  - Antin-Ozerkis, Danielle E.
A2  - Dela Cruz, Charles S.
A2  - Kotloff, Robert M.
A2  - Kotton, Camille Nelson
A2  - Pack, Allan I.
PY  - 2023
T2  - Fishman’s Pulmonary Diseases and Disorders, 6e
AB  - Pulmonary  alveolar  proteinosis  (PAP)  is  a  rare  syndrome  characterized  by  myeloid  cell  dysfunction,  progressive  pulmonary  surfactant  accumulation,  hypoxemia,  innate  immunodeficiency,  and  in  some  individuals,  serious  infections,  pulmonary  fibrosis,  respiratory  failure,  and  death.1  While  PAP-causing  diseases  are  clinically  and  mechanically  heterogeneous,  they  are  usefully  categorized  based  on  pathogenesis  into  primary,  secondary,  and  congenital  PAP.  Primary  PAP  is  caused  by  impaired  granulocyte-macrophage  colony-stimulating  factor  (GM-CSF)-dependent  surfactant  clearance  by  alveolar  macrophages  and  accounts  for  about  90%  of  cases.2  Secondary  PAP  occurs  as  a  consequence  of  a  comorbid  condition  that  impairs  surfactant  clearance  by  alveolar  macrophages  and  accounts  for  about  5%  to  10%  of  cases.3  Congenital  PAP—more  appropriately,  surfactant  metabolic  dysfunction  disorders—constitutes  a  clinically  distinct  and  heterogeneous  group  of  genetic  diseases  associated  with  acute  respiratory  failure  at  birth  from  surfactant  deficiency/dysfunction  or  progressive  pulmonary  fibrosis,  production  of  abnormal  surfactant,  and  varying  degrees  of  surfactant  accumulation  (hence,  their  consideration  as  PAP);  these  disorders  account  for  less  than  5%  of  cases.4  Importantly,  research  on  the  pathogenesis  of  primary  PAP  identified  a  critical  role  for  GM-CSF  in  surfactant  homeostasis,  alveolar  stability,  lung  function,  and  host  defense.  Because  of  its  increased  occurrence  and  greater  research  attention,  primary  PAP  is  the  primary  focus  of  this  chapter;  data  for  secondary  and  congenital  PAP  are  provided  when  available.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accessmedicine.mhmedical.com/content.aspx?aid=1195007479
ER  -